中南医学科学杂志2025，Vol.53Issue(2)：221-225,5.DOI:10.15972/j.cnki.43-1509/r.2025.02.007
JAK2/p-JAK2在ALS-SOD1-G93A转基因小鼠发病及进展中的表达
Expression of JAK2/p-JAK2 in the pathogenesis and progression of ALS-SOD1-G93A mice
摘要
Abstract
Aim To observe the role of Janus kinase(JAK2)/phosphorylated JAK2(p-JAK2)in the pathogenesis and pro-gression of amyotrophic lateral sclerosis(ALS)in superoxide dismutase 1(SOD1)G93A transgenic mice.Methods ALS-SOD1-G93A transgenic mice carrying the human SOD1 mutation gene were generated.Motor function was assessed using a motor deficit sco-ring system,and behavioral observations were conducted.Subsequently,six mice each at 30,60,and 90 days of age,at the onset stage of ALS,at the end-stage of ALS of SOD1-G93A transgenic mice,and 130-day-old non-transgenic(non-Tg)mice were randomly selected and assigned to the 30-day,60-day,90-day,onset,end-stage,and non-Tg groups,respectively.The lumbar spinal cord enlargement region was harvested from each group.Western blotting and immunohistochemical assays were performed to evaluate the expression levels of JAK2 and p-JAK2 proteins.Results Immunohistochemical results showed that the positive expression of JAK2 was weak in the spinal cord and lumbar enlargement of ALS-SOD1-G93A transgenic mice at different stages and non-Tg mice.Weak positive expression with cellular morphology was observed in the 30-d group,the 60-d group and the 90-d group,while the ex-pression was slightly increased in the onset group and the end-stage group.p-JAK2 positive expression was weaker in the non-Tg group,the 30-d group,the 60-d group and the 90-d group,which mainly concentrated in the cytoplasm of motor neurons in the gray matter of anterior spinal cord horn.However,there were few glial cells with strong p-JAK2 expression in the 30-d group,the 60-d group and the 90-d group,which affected the white matter.As age of the mice increased,glial cells with strong p-JAK2 positive ex-pression increased.In the onset group and the end stage group,p-JAK2 positive expression was significantly enhanced and concen-trated in proliferating glial cells.It affected gray and white matter areas of the lumbar spinal cord extensively.Western blotting re-sults showed that the expression levels of JAK2 and p-JAK2 in the end-stage group were significantly higher than those in the non-Tg group,30-d group,60-d group groups(P＜0.05).Conclusion In the pathogenesis of ALS-SOD1-G93A transgenic mice,the expression of JAK2 and p-JAK2 is significantly upregulated.After onset of disease,p-JAK2 is mainly expressed in proliferating glial cells of ALS-SOD1-G93A transgenic mice,indicating that JAK2/p-JAK2 plays an important role in the pathogenesis of ALS.关键词
ALS/JAK2/p-JAK2/SOD1/神经变性疾病/ALS-SOD1-G93A转基因小鼠Key words
ALS/JAK2/P-JAK2/neurodegenerative disease/ALS-SOD1-G93A mouse分类
医药卫生引用本文复制引用
齐银矿,文岚,余建萍,李堃毅,刘勇..JAK2/p-JAK2在ALS-SOD1-G93A转基因小鼠发病及进展中的表达[J].中南医学科学杂志,2025,53(2):221-225,5.基金项目
四川省医学科研青年创新课题计划(Q20047) （Q20047）
